We present a case of a young woman, age 34, who presented with recurrent Lyme neuroborreliosis (LNB). Her clinical profile consisted of a rare combination of two thirdstage manifestations, namely, progressive encephalomyelitis and peripheral neuritis, in both bouts of the disease. The epidemiological data were controversial, as she reported a tick bite only two months prior to the onset of symptoms. Negative magnetic resonance imaging (MRI) results excluded multiple sclerosis, vascular causes and tumours. Serological tests confirmed the Bb infection in the recommended twostep serological approach consisting of an enzyme-linked immunosorbent assay (ELISA) test for Borrelia burgdorferi (Bb) immunoglobulin G (IgG) and immunoglobulin M (IgM) and a Western blot (WB) as confirmatory analysis in the blood. Another controversial issue is the lack of pleocytosis. Atypical findings in our patient can be explained by a possible rare genotype of Bb. After treatment with oral doxycycline, she made an apparent remission, but after three months, she had another episode with signs of central and peripheral nervous system involvement, increased Bb antibodies and white matter changes on the MRI. This time, she was treated with intravenous ceftriaxone, 2 grams daily for four weeks. She showed no signs of LNB, both clinically and serologically, during a follow up lasting about a year. This case emphasises the importance of the clinical and serological findings and the use of ceftriaxone as the first line of treatment in LNB.